Congenital pseudarthrosis of the tibia treated with a combination of Ilizarov’s technique and intramedullary rodding : 15 cases followed for mean 4.5 years

Agashe, Mandar Vikas,Song, Sang-Heon,Refai, Mohammad Ahmed,Park, Kwang-Won,Song, Hae-Ryong Informa Healthcare 2012 Acta orthopaedica Vol.83 No.5

<P><B>Background and purpose</B></P><P> Ilizarov’s technique and intramedullary rodding have often been used individually in congenital pseudarthrosis of the tibia. In this series, we attempted to combine the advantages of both methods while minimizing the complications.</P><P><B>Methods</B></P><P> We reviewed 15 cases of congenital pseudoarthrosis of the tibia (CPT) who were treated with a combination of Ilizarov’s apparatus and antegrade intramedullary nailing between 2003 and 2008. The mean age at surgery was 7.5 (3–12) years and the mean limb length discrepancy was 2.5 (1.5–5) cm. At a mean follow-up time of 4.5 (1.6–7.2) years after the index surgery, the patients were evaluated clinically and radiographically for ankle function (AOFAS score) and for malalignment, signs of union, limb length discrepancy, and complications.</P><P><B>Results</B></P><P> 14 patients achieved union, in 6 patients primary union and in 8 patients after secondary procedures. The AOFAS score improved from a preoperative mean of 40 (20–57) to 64 (47–75). The main complication was refracture in 1 patient, and non-union in 1 patient.</P><P><B>Interpretation</B></P><P> The combination of the Ilizarov technique and conventional antegrade intramedullary nailing was successful in achieving union with few complications, though this should be shown in long-term studies lasting until skeletal maturity.</P>

Physeal growth arrest after tibial lengthening in achondroplasia : 23 children followed to skeletal maturity

Song, Sang-Heon,Agashe, Mandar Vikas,Huh, Young-Jae,Hwang, Soon-Young,Song, Hae-Ryong Informa Healthcare 2012 Acta orthopaedica Vol.83 No.3

<P><B>Background and purpose</B></P><P>Bilateral tibial lengthening has become one of the standard treatments for upper segment-lower segment disproportion and to improve quality of life in achondroplasia. We determined the effect of tibial lengthening on the tibial physis and compared tibial growth that occurred at the physis with that in non-operated patients with acondroplasia.</P><P><B>Methods</B></P><P>We performed a retrospective analysis of serial radiographs until skeletal maturity in 23 achondroplasia patients who underwent bilateral tibial lengthening before skeletal maturity (lengthening group L) and 12 achondroplasia patients of similar height and age who did not undergo tibial lengthening (control group C). The mean amount of lengthening of tibia in group L was 9.2 cm (lengthening percentage: 60%) and the mean age at the time of lengthening was 8.2 years. The mean duration of follow-up was 9.8 years.</P><P><B>Results</B></P><P>Skeletal maturity (fusion of physis) occurred at 15.2 years in group L and at 16.0 years in group C. The actual length of tibia (without distraction) at skeletal maturity was 238 mm in group L and 277 mm in group C (p = 0.03). The mean growth rates showed a decrease in group L relative to group C from about 2 years after surgery. Physeal closure was most pronounced on the anterolateral proximal tibial physis, with relative preservation of the distal physis.</P><P><B>Interpretation</B></P><P>Our findings indicate that physeal growth rate can be disturbed after tibial lengthening in achondroplasia, and a close watch should be kept for such an occurrence—especially when lengthening of more than 50% is attempted.</P>

Comparison between upper and lower limb lengthening in patients with achondroplasia : a retrospective study

Kim, S-J.,Agashe, M. V.,Song, S-H.,Choi, H-J.,Lee, H.,Song, H-R. British Editorial Society of Bone and Joint Surger 2012 The Bone & Joint Journal Vol.94 No.1

Paravertebral Ligament Ossification in Vitamin D–Resistant Rickets: Incidence, Clinical Significance, and Genetic Evaluation

Lee, Suk-Ha,Agashe, Mandar V.,Suh, Seung-Woo,Yoon, Yong-Cheol,Song, Sang-Heon,Yang, Jae-Hyuk,Lee, Hanna,Song, Hae-Ryong Lippincott Williams & Wilkins 2012 Spine Vol.37 No.13

STUDY DESIGN.: A retrospective observational study. OBJECTIVE.: The objective of this study was to analyze the cases of paravertebral ossification in vitamin D–resistant rickets (VDRR) and elucidate its incidence and clinical significance, and report specific genetic mutations uniquely associated with this phenomenon. SUMMARY OF BACKGROUND DATA.: Paravertebral ligament ossification has been described in VDRR in a few cases previously. However, supraspinous and interspinous ligament ossification has so far not been described to occur in association with VDRR. We reviewed 6 cases of paravertebral ligament ossification with the added feature of supraspinous and interspinous ligament calcification. METHODS.: Forty-four patients with clinical, radiological, and biochemical diagnoses of VDRR, who were positive for mutations in the PHEX gene, were screened for the presence of paravertebral ossification with plain radiographs and computed tomography/magnetic resonance imaging if necessary. This was correlated with the severity of disease, and we looked for specific genetic mutations. RESULTS.: Six patients had paravertebral ligament ossification, which included supraspinous and interspinous ligament ossification. These patients had a more severe systemic disease, with all patients requiring at least 1 surgery for deformity correction of the limbs, with a total of 10 surgical procedures performed in these 6 patients. c.1601C>T, c.1699C>T, c.1363G>T, and c.466_467insAC were the genetic mutations associated with these cases. CONCLUSION.: The spinal changes with paravertebral ligament ossification in VDRR were described in this study. Four different gene mutations related to the PHEX gene were detected in them. The presence of these changes was suggestive of a more severe systemic disease.

Is Bilateral Lower Limb Lengthening Appropriate for Achondroplasia?: Midterm Analysis of the Complications and Quality of Life

Kim, Seung-Ju,Balce, Gracia Cielo,Agashe, Mandar Vikas,Song, Sang-Heon,Song, Hae-Ryong Ovid Technologies (Wolters Kluwer) - Lippincott Wi 2012 Clinical orthopaedics and related research Vol.470 No.2

Growth disturbance after lengthening of the lower limb and quantitative assessment of physeal closure in skeletally immature patients with achondroplasia

Song, S. H.,Kim, S. E.,Agashe, M. V.,Lee, H.,Refai, M. A.,Park, Y. E.,Choi, H. J.,Park, J. H.,Song, H. R. British Editorial Society of Bone and Joint Surger 2012 The Bone & Joint Journal Vol.94 No.4

<P>This study evaluated the effect of limb lengthening on longitudinal growth in patients with achondroplasia. Growth of the lower extremity was assessed retrospectively by serial radiographs in 35 skeletally immature patients with achondroplasia who underwent bilateral limb lengthening (Group 1), and in 12 skeletally immature patients with achondroplasia who did not (Group 2). In Group 1, 23 patients underwent only tibial lengthening (Group 1a) and 12 patients underwent tibial and femoral lengthening sequentially (Group 1b). The mean lengthening in the tibia was 9.2 cm (59.5%) in Group 1a, and 9.0 cm (58.2%) in the tibia and 10.2 cm (54.3%) in the femur in Group 1b. The mean follow-up was 9.3 years (8.6 to 10.3). The final mean total length of lower extremity in Group 1a was 526.6 mm (501.3?to 552.9) at the time of skeletal maturity and 610.1 mm (577.6 to 638.6) in Group 1b, compared with 457.0 mm (411.7 to 502.3) in Group 2. However, the mean actual length, representing the length solely grown from the physis without the length of distraction, showed that there was a significant disturbance of growth after limb lengthening. In Group 1a, a mean decrease of 22.4 mm (21.3 to 23.1) (4.9%) was observed in the actual limb length when compared with Group 2, and a greater mean decrease of 38.9 mm (37.2 to 40.8) (8.5%) was observed in Group 1b when compared with Group 2 at skeletal maturity. In Group 1, the mean actual limb length was 16.5 mm (15.8 to 17.2) (3.6%) shorter in Group 1b when compared with Group 1a at the time of skeletal maturity. Premature physeal closure was seen mostly in the proximal tibia and the distal femur with relative preservation of proximal femur and distal tibia. We suggest that significant disturbance of growth can occur after extensive limb lengthening in patients with achondroplasia, and therefore, this should be included in pre-operative counselling of these patients and their parents.</P>

Immune Characterization of Bone Marrow-Derived Models of Mucosal and Connective Tissue Mast Cells

Sara Benedé,Evan Cody,Charuta Agashe,M. Cecilia Berin 대한천식알레르기학회 2018 Allergy, Asthma & Immunology Research Vol.10 No.3

Purpose: It is well appreciated that mast cells (MCs) demonstrate tissue-specific imprinting, with different biochemical and functional properties between connective tissue MCs (CTMCs) and mucosal MCs (MMCs). Although in vitro systems have been developed to model these different subsets, there has been limited investigation into the functional characteristics of the 2 major MC subsets. Here, we report the immunologic characterization of 2 MCs subsets developed in vitro from bone marrow progenitors modeling MMCs and CTMCs. Methods: We grew bone marrow for 4 weeks in the presence of transforming growth factor (TGF)-β, interleukin (IL)-9, IL-3, and stem cell factor (SCF) to generate MMCs, and IL-4, IL-3, and SCF to generate CTMCs. Results: CTMCs and MMCs differed in growth rate and protease content, but their immune characteristics were remarkably similar. Both subsets responded to immunoglobulin E (IgE)-mediated activation with signaling, degranulation, and inflammatory cytokine release, although differences between subsets were noted in IL-10. CTMCs and MMCs showed a similar toll-like receptor (TLR) expression profile, dominated by expression of TLR4, TLR6, or both subsets were responsive to lipopolysaccharide (LPS), but not poly(I:C). CTMCs and MMCs express receptors for IL-33 and thymic stromal lymphopoietin (TSLP), and respond to these cytokines alone or with modified activation in response to IgE cross-linking. Conclusions: The results of this paper show the immunologic characterization of bone marrow-derived MMCs and CTMCs, providing useful protocols for in vitro modeling of MC subsets.

New proposed clinico‐radiologic and molecular criteria in hypochondroplasia: <i>FGFR</i> 3 gene mutations are not the only cause of hypochondroplasia

Song, Sang‐,Heon,Balce, Gracia Cielo Estrera,Agashe, Mandar Vikas,Lee, Hanna,Hong, Suk‐,Joo,Park, Young‐,Eun,Kim, Sang‐,Gyun,Song, Hae‐,Ryong Wiley Subscription Services, Inc., A Wiley Company 2012 AMERICAN JOURNAL OF MEDICAL GENETICS PART A Vol.a158 No.10

<P><B>Abstract</B></P><P>We applied a comprehensive set of clinical and radiological criteria for the diagnosis of hypochondroplasia (HCH) in 160 patients with short stature 58 of whom were diagnosed to have HCH. Taking into account the genotypic and phenotypic variations in HCH, we conducted a study with these 58 patients and tested them for mutations in the fibroblast growth factor receptor 3 (<I>FGFR3</I>) and the short stature homeobox (<I>SHOX</I>) gene. We characterized the phenotypes by clinical and radiologic findings. In the patients with HCH, 19 were included in Group I (<I>FGFR</I>3 mutations—mutations of definite significance), and 39 were in Group II (6 <I>SHOX</I> mutations and 33 negative for disease‐causing FGFR3 mutations). The clinical findings were similar in two groups regardless of the presence or absence of mutations. More than 95% of the patients had mesomelic proportions. In Group I, the radiological findings of mesomelia of upper and lower limbs and, L1/L4 ratio in anterior–posterior and lateral view were more typical than in Group II. This study proposes comprehensive clinico‐radiological criteria for the diagnosis of HCH, which would help in detecting the true incidence of this underdiagnosed condition. The presence of <I>SHOX</I> mutations suggest genotypic–phenotypic overlap between HCH and Leri–Weill dyschondrosteosis, though further investigation is needed to effectively elucidate the importance of these mutations. Also, the 56.9% of HCH patients with negative mutations for <I>FGFR3</I> suggests that there are other undiscovered gene mutations associated with this phenotypic entity. © 2012 Wiley Periodicals, Inc.</P>

Influence of Preparation Parameters on Characteristics of Zirconia - Pillared Clay using Ultrasonic Technique and its Catalytic Performance in Phenol Hydroxylation Reaction

Awate, Shobhana Vaman,Waghmode, Suresh Babasaheb,Kashinath Rangu,Patil, Agashe, Mangala Shripad,Joshi, Praphulla Narahar 한국화학공학회 2001 Korean Journal of Chemical Engineering Vol.18 No.2

Ultrasonication method was employed for preparation of zirconia-pillared montmorillonite (Zr-PILC) by using polyhydroxy zirconium cation. The influences of various preparation parameters such as pH, concentration of pillaring agent, aging period on physico-chemical properties of Zr-PILCs are reported. The characterization was done by using different characterizing tools such as powder X-ray diffraction (XRD), thermal analysis (TG/DTA), Fourier transform infrared spectroscopy (FITR), X-ray photo-electron spectroscopy (XPS), X-ray fluorescence spectroscopy (XRF) and surface area measurements. Effective pillaring was obtained in the pH range 2.0-2.8 at reduced preparation time by using ultrasonication. Ultrasonication, especially at higher pH and concentration of pillaring agent with no aging after ultrasonic agitation of the clay slurry was found to increase zirconium content and specific BET surface area leaving no scope for remarkable increase in d001 spacing. The presence of -OH groups attached to Zr has been revealed by pyridine-adsorbed FTIR, TG/DTA and XPS techniques. The acidic character, ease of accessibility and good dispersion of active sites in Zr-PILC were found to be controlling factors for the challenging activity in hydroxylation reaction of phenol. Probably, this first report on the use of Zr-PILC in hydroxylation of phenol and its preparation by employing ultrasonication technique may attract great attention in the catalysis area of academic and industrial importance.

Role of Cytokines in Genesis, Progression and Prognosis of Cervical Cancer

Paradkar, Prajakta Hemant,Joshi, Jayashree Vinay,Mertia, Priyanka Nirmalsingh,Agashe, Shubhada Vidyadhar,Vaidya, Rama Ashok Asian Pacific Journal of Cancer Prevention 2014 Asian Pacific journal of cancer prevention Vol.15 No.9

Cytokine research is currently at the forefront in cancer research. Deciphering the functions of these multiple small molecules, discovered within the cell and in intercellular spaces, with their abundance and pleotrophism, was initially a great challenge. Advances in analytical chemistry and molecular biology have made it possible to unravel the pathophysiological functions of these polypeptides/proteins which are called interleukins, chemokines, monokines, lymphokines and growth factors. With more than 5 million women contracting cervical cancer every year this cancer is a major cause of mortality and morbidity the world over, particularly in the developing countries. In more than 95% of cases it is associated with human papilloma virus (HPV) infection which is persistent, particularly in those with a defective immune system. Although preventable, the mere magnitude of prevalence of HPV in the world population makes it a dominating current health hazard. The discovery of cytokine dysregulation in cervical cancer has spurted investigation into the possibility of using them as biomarkers in the early diagnosis of cases at high risk of developing cancer. Their critical role in carcinogenesis and progression of cervical cancer is now being revealed to a great extent. From diagnostics to prognosis, and now with a possible role in therapeutics and prevention of cervical cancer, the cytokines are being evaluated in all anticancer approaches. This review endeavours to capture the essence of the astonishing journey of cytokine research in cervical neoplasia.